PSEUDOMYXOMA PERITONEI - LONG-TERM PATIENT SURVIVAL WITH AN AGGRESSIVE REGIONAL APPROACH

Objective The aims of this study were to analyze the natural history o f patients with pseudomyxoma peritonei (PMP), evaluate clinical and pa thologic variables as prognostic indicators, and review the authors' e xperience with different treatments. Summary Background Data PMP is an unusual form of intra-abdominal neoplasm that presents with large amo unts of extracellular mucin. Diffuse peritoneal spread occurs in most patients with PMP, and distant metastasis is infrequent. Debulking sur gery, radiation therapy (radioisotope and external beam), and chemothe rapy (both intraperitoneal and systemic) have all been advocated for o ptional patient management, but the variability of patients studied, t he small patient numbers, and the prolonged course of this disease mak e the evaluation of results difficult. Methods Fifty-six patients were treated for PMP at the Mayo Clinic between 1957 and 1983. The data we re collected retrospectively. Univariate (log-rank test) and multivari ate (Cox regression model) analyses were performed for disease recurre nce and patient survival. Results Most patients with PMP had carcinoma s of the appendix (52%) or ovary (34%). All gross tumor could be remov ed only in the 34% of patients with limited disease. Although tumor pr ogression occurred in 76% of patients, the 1-, 5-, and 10-year surviva l rates were 98%, 53%, and 32%, respectively. Adverse predictors of pa tient survival included weight loss (p = 0.001), abdominal distention (p = 0.004), use of systemic chemotherapy (p = 0.005), diffuse disease (p = 0.038), and invasion of other organs (p = 0.04). Intraperitoneal chemotherapy (p = 0.009) and radioisotopes (p = 0.0043) both were eff ective in prolonging the recurrence time of symptomatic PMP. Conclusio ns Although PMP is an indolent disease, aggressive surgical debulking followed by intraperitoneal radioisotopes and/or chemotherapy should b e considered because of the diffuse peritoneal involvement.